The Nikolsky sign in staphylococcal scalded skin syndrome

Editor,—Ladhani and Evans, in their account of staphylococcal scalded skin syndrome (SSSS),1accurately describe “large fluid-filled bullae which quickly rupture on slightest pressure” but incorrectly call this the
Nikolsky sign. Fragility of blisters merely reflects their superficial position in the epidermis. The Nikolsky sign is dislodgement
of intact superficial epidermis by a shearing force, indicating a plane of cleavage in the skin. The defect may be due to
staphylococcal toxin as in SSSS, or to epidermal antibodies as in pemphigus. In his thesis on pemphigus in 1895, the Russian
dermatologist Pyotr Vasilyevich Nikolsky (1858–1940) described “a weakening relationship and contact among the epidermal layers
even in places between lesions on the seemingly unaffected skin”. He described three ways of eliciting the sign: (1) the stratum
corneum, when pulled, can be stripped off over large areas, and it is possible to displace the stratum corneum of (2) healed
skin and (3) healthy uninvolved skin by rubbing. Nikolsky himself gave some credit for determining this sign to his teacher
Professor Stoukavenkow at the University of Kiev (1884–1935).2

Recently, a 14 month old girl under our care dramatically demonstrated the Nikolsky sign in SSSS. Three days after a minor
skin injury on her left thumb which became infected, she developed irritability, coryza, and spreading erythema starting on
the face and neck. She presented the next day with exquisitely tender red skin, and within 24 hours large areas of superficial
epidermis separated, particularly under the edges of clothing, and in the axillae. Sheets of epidermis peeled back revealing
painful raw areas (fig 1). Sparing of the mucosae excluded the alternative diagnoses of toxic epidermal necrolysis and Stevens-Johnson syndrome. She
recovered promptly with intravenous flucloxacillin. As would be expected from the superficial level of the epidermal split
in SSSS there was no scarring.